Background: Cushings syndrome in children and adolescents is rare. Its clinical and biological symptoms are severe with a significant impact on growth and puberty and poor prognosis.
Objective and hypotheses: Report clinical, etiological and evolutionary characteristics of Cushings syndrome in children and adolescents.
Method: This is a retrospective study of 45 children and adolescents with Cushings syndrome hospitalised at the department of endocrinology between January 1988 and December 2014. We have evaluated the clinical, biological, aetiological and evolutionary Cushings syndrome in children and adolescents.
Results: The mean age at diagnosis was 11.4 years (619), 2/3 of them will have an age >10 years. The sex ratio F/G is 2.25. Clinical presentation was characteristic and significant in all cases. We observed complications In 22.5% (Diabetes mellitus 40% hypertension 30% dyslipidaemia 30% osteoporosis 20%). Aetiologies were iatrogenic causes 60%, Cushing diseases 33.3% adrenal corticosurrenaloma 6.6%. The transsphenoidal surgery has allowed a sustained remission of Cushings disease in 30%. In 70% of relapse revision surgery + radiotherapy resulted in a adrenocorticotropic insufficiency. Adrenal surgery of carcinoma resulted in a good evolution. After a mean follow-up of six years, a Cushing disease recurrence was observed in 15%.
Conclusion: The clinical and biological Cushings syndrome in children and adolescents is often severe and complicated. Aetiological, Cushings disease is more common in adolescents while malignant adrenal tumour most is often the prerogative of younger children. Their management is tricky with initial failures and high incidence of recurrence requiring monitoring the long course. Concertation between doctors endocrinologists, paediatricians, radiologists and surgeons is essential.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology